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At least 19 recordsLinked to original sources
PubMed1976

The long-term prognosis infantile spasms--the present condition of cases of infantile spasms followed in school age.

A follow-up study has been made of 25 cases with infantile spasms, all of whom were six years old or more at review. Only four (16%) out of 25 cases made a full recovery and attended normal school. Spasms ceased in 96% of all cases, but fits other than spasms (grand mal, tonic seizure, atonic seizure, myoclonic seizure, atypical absence and psychomotor seizure) occurred subsequently in 11 cases (44%). The EEG became normal in two cases (8%), but still showed modified hypsarhythmia in three cases (12%), "epileptic non-hypsarhythmic" discharges in 17 cases (68%) and non-specific abnormalities in three cases (12%). The important factors associated with good prognosis were normal development before the onset of spasms, late onset (seven months old or over) and short duration of spasms, the absence of other types of fit following spasms and lack of neurological abnormality. A bad prognosis was associated with abnormal development prior to the onset of spasms, early onset and long duration of spasms, the presence of other types of fit following spasms and evidence of any neyrological abnormality This follow-up may confirm that the therapy with ACTH-A has no significant effect on final mental state.

Adolescent↗
PubMed1977

Genetic study of infantile spasm with hypsarrhythmia.

Infantile spasms (IS) appear to be a distinct syndrome complicated by heterogeneous etiology. Family data support a multifactorial model involving a polygenic determination of susceptibility to IS but requiring environmental factors such as anoxia, birth trauma, or immunization to precipitate seizures. The empiric recurrence risk among siblings was estimated to be 15 +/- 3 and for all first degree relatives as 7 +/- 5 per 1,000. These risks should be interpreted with caution since possible heterogeneity of IS may result in the occurrence of families in which cases are presumably totally environmental, and other rare families which may be segregating for an autosomal recessive disorder. Careful review of involved medical and family histories and a thorough physical examination should permit discrimination among these possibilities.

Epilepsy↗
PubMed1977

Critical evaluation of the role of immunization as an etiological factor of infantile spasms.

One hundred and ten cases of infantile spasms with detailed information about immunization available were selected as the masterial to evaluate the significance of immunization as an etiological factor of infantile spasms. In 80% of the cases, immunization could not be considered to have any relation with causation of infantile spasms, because 44 cases (40%) never had innoculations as yet, and other 44 cases (40%) had been immunized by some vaccines over one month before or after the onset of the disease. The remaining 22 cases, in whom immunization had been performed within one month before the onset of the disease, constituted the candidates for further study. The age of onset of the disease of the candidate group ranged from 3 to 9 months of age with peak incidence at 4 to 5 months old, exactly identical with that of patients with infantile spasms in general. The kind of vaccines concerned included DPT triple vaccines in 15 cases, smallpox vaccine in 4, antipolio live vaccine in 1, anti-Japanese encephalitis vaccine in 2. Causal relationship of immunization with infantile spasms in each patient was evaluated on the basis of the following three apsects: 1. Any other causal factors except immunization should be absent. 2. Psychomotor development before onset of the disease should be normal. 3. Interval from immunization to onset of the disease should be lied: a) Within 48 hours, in the case of pertussis vaccine, b) within 18 days, in the case of smallpox vaccine or anti-Japanese encephalitis vaccine or antipolio vaccine. Only five cases (4.8% of the total) were able to classify into the compatible group which should fulfill all the above three criteria. The small figure may easily be explicable on the assumption that the natural onset of spasm is chronologically superposed by chance over immunizations which have to be done within the first year of life.

Age Factors↗
PubMed1978

Infantile spasms and HLA antigens.

21 new cases of infantile spasms were reported in 1976 from paediatric departments in Denmark. The connection between infantile spasms and the Lennox-Gastaut syndrome is mentioned, because of reports of a significantly higher incidence of HLA-B7 in children with Lennox-Gastaut syndrome. The HLA antigen distribution in 19 of the 21 children was compared with that of 1967 healthy adults. No difference in the HLA antigens was demonstrated between children with infantile spasms and controls, whether in the material as a whole, or in the cryptogenic or symptomatic groups. However HLA typing of children with infantile spasms should continue in the search for a potential genetic marker in this grave disease, particularly in view of the reported high incidence of HLA-B7 in children with the Lennox-Gastaut syndrome.

Female↗
PubMed1975

Infantile spasms associated with histidinemia.

A case of infantile spasms associated with histidinemia is presented. Histidinemia was well-documented through biochemical assays. The patient was treated with the standard anticonvulsant regimen for infantile spasms, as well as an elimination diet for histidinemia. Despite low levels of histidine and adequate anticonvulsant therapy, the child continues to have seizures and is markedly retarded. The natural history of infantile spasms and its possible association with histidinemia is discussed.

Amino Acid Metabolism, Inborn Errors↗
PubMed1976

Congenital retarded myelinization in a new-born child with infantile spasms.

A new-born child with frequent infantile spasms was investigated. In a cachectic state it died at the age of 11 months. Biopsy of the cerebral cortex revealed a retarded myelinization; at autopsy this slight myelinization was found again. The ganglioside pattern suggested a developmental stage of approximately 3 months before birth. This is a case with a congenital failure of myelinization.

Autopsy↗
PubMed1979

A controlled study of prednisone therapy in infantile spasms.

A controlled study of 12 patients with infantile spasms was performed to determine the effectiveness of prednisone treatment. Patients were monitored serially, using a time-synchronized polygraphic and video system. Three patients (25%) showed prompt reduction in seizure frequency and normalization of the EEG after institution of treatment. The remaining patients showed no improvement in seizure frequency or significant change in the EEG.

Cerebral Hemorrhage↗
PubMed1977

Infantile spasms and early immunization against whooping cough. Danish survey from 1970 to 1975.

113 cases of infantile spasms were diagnosed in the period 1 April 1970 to 31 March 1975 after Denmark changed her immunization programme. Previously whooping cough vaccine was given as the triple combination at 5, 6, and 15 months of age. From 1 April 1970 it was given as a monovalent vaccine at 5 and 9 weeks of age and at 10 months of age. A comparison with previous material of 86 cases of infantile spasms from 1957 to 1967 showed no change in the age at onset. Though there may be an occasional connection between immunization and infantile spasms, the most important factor is a time-coincidence between any immunization given at a certain age with the neurological disorders which are natural for that age.

Denmark↗
PubMed1979

Choroid plexus papilloma and infantile spasms.

A 7-month-old infant had the infantile spasm syndrome, consisting of flexor and extensor spasms, developmental delay, and hypsarrhythmia. Corticotropin produced delay, and hypsarrhythmia. Corticotropin produced improvement in the clinical symptoms and reverted the generalized electroencephalographic abnormalities to more focal ones. Removal of a choroid plexus papilloma of the left lateral ventricle was followed by clinical recovery. One year later the child was normal developmentally and neurologically and was seizure free on anticonvulsant therapy.

Cerebral Ventricle Neoplasms↗
PubMed1979

The ocular findings in infantile spasms.

The results of this study suggest that a careful ophthalmoscopic examination of children with infantile spasms will help to establish an etiologic diagnosis in at least 20% of cases. The two clinical entities identifiable by this examination are tuberous sclerosis and Aicardi's syndrome. It is emphasized that the retinal hamartomas of tuberous sclerosis seen in infancy are flat, semitransparent, and small. The mulberry-like lesion is distinctly unusual in this age group. The essential ocular feature of Aicardi's syndrome is a chorioretinopathy of the posterior pole. This may be accompanied by microphthalmus, persistent pupillary remnant, and colobomas of the optic nerve and choroid. Diagnosis of either of these two entities is helpful in establishing the prognosis for a child with infantile spasms since both of these disorders usually lead to severe neurologic handicap and premature death. Moreover, genetic counseling is essential for the family suffering with this tragic disorder. Tuberous sclerosis is inherited as an irregular dominant trait. The genetic interpretation of sporadic cases, which are in the majority, is difficult due to the presence of unrecognized incomplete or "fruste" forms within families. On the other hand, Aicardi's syndrome has never been reported to be familial and future pregnancies can be undertaken without fear of producing another child with infantile spasms.

Child, Preschool↗
PubMed1978

Quantitative analysis and characterization of infantile spasms.

An effective, time-synchronized monitoring system for the study of patients with infantile spasms has been developed. This system utilizes concurrent graphically recorded data, including EEG, body movement via triaxial accelerometry, respiration, electrocardiogram, electromyogram, electro-oculogram, and galvanic skin response. These data, when evaluated in conjunction with a closed-circuit TV recording of the patient, greatly facilitate the differentiation of seizures from nonictal activity and permit the characterization and quantification of the behavioral, motor, and autonomic phenomena intrinsically associated with infantile spasms.

Electrocardiography↗
PubMed1979

Long-term prognosis of patients with infantile spasms following ACTH therapy.

The influence of ACTH on the prognosis of patients with infantile spasms remains controversial. We have examined retrospectively the long-term benefits of initially successful ACTH therapy in patients treated at this institution between 1961 and 1974. Individuals with equivocal or minimal improvement during ACTH therapy were excluded from this study. Eighteen affected infants showed a favorable early response consisting of cessation of seizures for at least 3 weeks during ACTH therapy and concurrent disappearance of the hypsarhythmic EEG pattern. Modal age at last follow-up was 5 years (range, 15 months to 16 years). Infantile spasms recurred in 7 patients (39%), and 8 patients subsequently had other seizure types. All epileptiform (spike) activity disappeared from the EEGs of 8 patients during ACTH therapy, but in 4 of these cases epileptiform activity was present in later tracings. In the remaining 10 patients the hypsarhythmic pattern disappeared in association with ACTH therapy, but the EEG remained epileptiform (often only in sleep). Later EEGs were free of epileptiform activity in 5 of the 10 patients whose tracings contained spike discharges in the early follow-up period. Four patients (22%) were seizure free and without intellectual impairment when last evaluated.

Adrenocorticotropic Hormone↗
PubMed1979

Polygraphic study during whole night sleep in infantile spasms.

The whole night EEG were polygraphically recorded and analyzed in 9 patients with infantile spasms prior to ACTH therapy. The subjects were divided into two groups, favorable and unfavorable, depending upon the response to the ACTH therapy. (1) Among the unfavorable group, the deep sleep stage was not observed; while the light sleep stage tended to dominate. (2) REM sleep period was noted less among the unfavorable than among the favorable group. REM density also tended to be lower among the unfavorable group. (3) Of the 4 unfavorable cases, 2 did not manifest REM sleep at all. Of the remaining 2, 1 had a remarkably long REM interval period. Even among the favorable cases, REM sleep tended to be short and appear frequently. (4) The period of muscle atonia during NREM sleep was markedly prolonged in all cases. (5) Body movements of both types (gross and twitch) were less frequent comparing to those of normal younger children, more remarkably in unfavorable cases. From the above findings, a disorder of the pontine reticular formation would be suggested in cases of infantile spasms. Reduction of body movements at each sleep stage might indicate abnormalities of monoamine metabolism in the brain stem of patients with this condition.

Adrenocorticotropic Hormone↗
PubMed1979

A study on Sturge-Weber syndrome. Report of a case associated with infantile spasms and electroencephalographic evolution in five cases.

A case of classical Sturge-Weber syndrome associated with infantile spasms (female infant, aged 4 months) was presented. The association of both conditions would be very rare since no similar case was found among 214 cases of Sturge-Weber syndrome and 1,180 cases of infantile spasms gathered from the literature, except for one who was reported by Millichap et al. Evolutional changes of electroencephalographic findings were followed up for about 3 years on average in 5 personal cases of Sturge-Weber syndrome. Unilateral depression of electrical activity in the cerebral hemisphere ipsilateral to the facial nevi was the constant finding. Focal spike discharges were noticed only in the contralateral hemisphere in 3 cases, only in the ipsilateral in 1, and in the bilateral in 1.

Angiomatosis↗
PubMed1977

The studies of the attacks of abnormal eye movement in a case of infantile spasms.

We observed the attacks of nystagmus in a case of infantile spasms and carried out overnight sleep polygraphy. The following findings were obtained: (1) Awake time was prolonged to 56% of TIB, while sleep time was markedly shortened to 44% of TIB. Also REM sleep time was reduced to 20% of SPT and deep slow sleep (stage N3) was absent. (2) Attacks of nystagmus were observed only during wakefulness. At first, fast wave bursts appeared in the right occipital area, and were followed by left-upward deviations of eyeballs, and then the attacks of counterclockwise right-downward rotatory nystagmus. Also the fast wave bursts were succeeded by spreading of fast waves to adjacent areas. At the same time, EMG exhibited elevated muscle action potentials of mentalis and biceps femoris. During the attacks of nystagmus, there was alteration of respiratory movements. In NREM sleep, both fast wave bursts and the attacks of nystagmus were not observed. In REM sleep, the fast wave bursts and eye movements were observed but the attacks of nystagmus did not appear. During the fast wave bursts, alteration of respiratory movements were observed. From the results of this study, it was presumed that the brain lesions of this case were extended with severe degree of damage in the great parts of brain containing cerebral cortex and brain stem.

Electroencephalography↗